Clinical profiles of Chinese patients with diffuse panbronchiolitis
a Department
of Medicine, b Department of Diagnostic Radiology, c Department of Pathology, d Queen Mary Hospital, The
University of Hong Kong, Pokfulam, Hong Kong Chest Disease
Research Institute, Kyoto University, Japan
Correspondence to: Dr K W T Tsang.
Received 24 July 1997; Returned to authors 18 December 1997; Revised version received 6 January 1998; Accepted for publication 7 January 1998
BACKGROUND
Diffuse panbronchiolitis (DPB),
characterised by progressive sinobronchial sepsis, is well
characterised in Japanese subjects but not in other ethnic groups. The
experience with DPB in seven Chinese patients is described and the
clinical profiles compared with those of Japanese subjects.
METHODSSeven Chinese patients (three women; mean
(SD) age 48(18.6) years, all never smokers) who attended a teaching
hospital centre and fulfilled the diagnostic criteria for DPB were
assessed prospectively for clinical, radiological, lung function,
microbiological, and other "characteristic" laboratory parameters.
RESULTSLung function assessment showed a typical
obstructive pattern (n = 5) and air trapping (n = 7). Typical
bronchiolar infiltration by lymphocytes and plasma cells and
accumulation of foamy macrophages in the intraluminal tissue were
detected in open lung biopsy specimens (n = 2). Chest radiographs and
high resolution computed tomographic scans revealed hyperinflation,
diffuse nodules, bronchial thickening and dilatation, peripheral
hypoattenuation, and bronchiolectasis. Radiological improvement,
manifest as a reduction in nodular density and bronchial thickening,
and persistence of other abnormalities such as air trapping were not
accurately depicted by the classical Nakata or Akira classifications.
The other "characteristic" features such as HLA-B54, IgG subclass
deficiency, raised CD4/CD8 T lymphocyte ratio, cold haemagglutinaemia,
raised IgA, IgG, and rheumatoid factor were not present. Treatment with
erythromycin led to excellent responses in symptoms, lung function
indices, and the radiological picture. A review of the non-Japanese
cases in the literature reveals that this absence of typical
"additional features" in DPB might also be applicable to
non-Japanese patients.
CONCLUSIONSWe report the only series of
non-Japanese Mongoloid patients with well characterised DPB who had
uncharacteristic investigation profiles. This experience should help
other clinicians in the investigation and management of DPB in
non-Japanese patients.
© 1998 by Thorax
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